Extralobar Sequestration Research Articles

Neonatal Outcomes of Multiple Congenital Thoracic Lesions

Introduction: Congenital pulmonary airway malformations (CPAM), intra- and extralobar bronchopulmonary sequestrations (iBPS/eBPS), CPAM-BPS hybrid lesions (HL), congenital lobar emphysema (CLE), bronchial atresia (BA), and foregut duplication cysts (FDC), collectively referred to as congenital thoracic lesions (CTL), are mostly solitary. Patients with multiple CTL are rare, and reports on such cases are scarce. To address this dearth, we analyzed a large multifocal CTL patient cohort. Methods: Retrospective chart review of patients born between September 1, 2013, and March 31, 2023, who underwent surgery for a CTL at our tertiary center. Patients with radiological and surgical diagnosis of multifocal CTL, defined as ≥2 CTL present in more than one lobe were included to record pre-, peri-, and postnatal patient characteristics. Results: Among 701 CTL patients, 74 (10.5%) had multiple CTL. CTL multifocality was prenatally recognized correctly in 8 (12.9%) patients. Most multiple CTL were right-sided, unilateral multilobar lesions (n = 33, 44%). Bilateral CTL were found in 9 (12.1%) patients. CPAM-CPAM lesions were the most prevalent CTL types (n = 36, 49%). Genetic syndromes were confirmed in 3 (4%) and additional congenital anomalies in 9 (12.9%) patients, 5 of those had multiple congenital anomalies. Of 49 (65%) patients with multilobar CTL, 25 (51%) underwent bilobectomy and 24 (49%) lung-sparing surgery. Length of stay was similar. Mortality was 5.4%. Conclusion: We report on the largest patient cohort with multiple CTL to date. Multiple CTL occurred in 1/10 patients with CTL, and only 12.9% were recognized prenatally. Lung-sparing surgery can be considered. Multiple additional congenital anomalies and genetic syndromes may be more common and genetic testing should be considered. Overall, outcomes in this patient population are favorable.

Association of Prenatal Congenital Lung Malformation Volume Ratio to Postnatal Computed Tomography Characteristics

IntroductionCongenital lung malformations (CLMs) are diverse and readily diagnosed on prenatal ultrasound. Postnatal computed tomography (CT) characteristics, including volume, are used in centers for the clinical decision-making of asymptomatic CLM. We aim to evaluate the relationship of prenatal CLM volume ratio (CVR) to postnatal CT characteristics by suspected prenatal diagnosis and postnatal radiological diagnosis. MethodsWe performed a single-center retrospective review of all patients evaluated with prenatally diagnosed CLM (May 2015-December 2022). Demographics, prenatal imaging findings at initial evaluation, and postnatal radiological diagnosis/imaging findings were analyzed with descriptive statistics. Pairwise correlation coefficient tests were performed to analyze the correlation between prenatal CVR and postnatal CT lesion size stratified by diagnosis. ResultsOf 123 patients referred and evaluated, suspected prenatal diagnoses consisted of 68 bronchial atresia (BA), 20 intralobar bronchopulmonary sequestration (iBPS), 20 extralobar bronchopulmonary sequestration (eBPS), and nine congenital pulmonary airway malformation (CPAM). Postnatal radiological diagnoses consisted of 53 BA, 22 iBPS, 14 eBPS, and 20 CPAM. Overall correlation coefficient of prenatal CVR to postnatal CT lesion size volume was 0.56. By suspected prenatal diagnosis, correlation coefficients were 0.61 (BA), 0.59 (iBPS), 0.29 (eBPS), and 0.51 (CPAM). For postnatal radiological diagnosis, correlation coefficients were 0.58 (BA), 0.56 (iBPS), 0.33 (eBPS), and 0.62 (CPAM). ConclusionsOur results indicate that initial CVR is overall consistent with the postnatal CT lesion size. This correlation is present in suspected prenatal diagnoses of BA and iBPS and postnatal radiological diagnoses of BA, iBPS, and CPAM. Additional studies analyzing long-term follow-up should be conducted to specify the safety of patients who undergo observation rather than surgical intervention.

Extralobar sequestration — not obvious radiologic finding. Illustrated overview. Case report

INTRODUCTION: Bronchopulmonary sequestration is a rare congenital pulmonary malformation. A sequestration is generally defined as non-functioning lung tissue that is not in normal continuity with the tracheobronchial tree and derives its blood supply from systemic vessels. OBJECTIVE: To define the main clinical and radiologic features of intra- and extralobar sequestration of the lung, to study vessel variability and to analyse case of not obvious CT images of intralobar sequestration that was diagnosed intraoperatively. MATERIALS AND METHODS: We analysed literature found in PubMed and ScienceDirect regarding lung sequestration since 1979. The case report of a patient with extralobar sequestration diagnosed intraoperatively.RESULTS: The main clinical and radiologic features of lung sequestration, challenges of differential diagnosis were summarised, including specificity of case report.DISCUSSION: Presence of aberrant vessel requires verification of pathology before operation due to high risk of haemorrhage. The leading role in the verification belongs to radiologic imaging, however in rare cases aberrant vessel may not be visualised due to small diameter or occlusion.CONCLUSION: The anatomy of aberrant vessels in sequestrations is extremely variable. In case report sequestration was seen in an adult. The pathology was not diagnosed prior to the operation due to failure of the vessel visualisation. Knowledge of possible diagnostic challenges decreases the risk of intraoperative complications.

Pediatric thoracoscopic lung resections: a comprehensive analysis of congenital lesion cases

Background Congenital lung lesions in pediatric patients may be managed conservatively or by video assisted thoracoscopic surgery (VATS). This study aimed to determine the complications after VATS for congenital lung lesions in children. Methods All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Children undergoing a primary open lobectomy or a resection other than a congenital lung lesion were excluded. Both early (<30 days) and late postoperative pulmonary complications were determined. The primary endpoint was postoperative complications within 30 days. Results In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, fewer resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof(p = 0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. During follow-up a pneumonia occurred in 11 patients. Conclusion There seems to be a shift towards delayed surgery with an increase in symptomatic congenital lung malformations, which might lead to an increase in postoperative complications

Absorbable Clips Applied in Thoracoscopic Anatomical Lung Resection in Younger Children.

This study aims to evaluate the feasibility and safety of a 5-mm absorbable clips applied in thoracoscopic anatomical lung resection in younger children. Demographic data and intra- and postoperative parameters of the two groups (Abs-o-lock® group and Hem-o-lok® group) were reviewed. In the Abs-o-lock® group, 5-mm absorbable clips were used in thoracoscopic anatomical lung resection on all patients from January 2020 to March 2021. In the Hem-o-lok® group, 5-mm Hem-o-lok® clips were used from January to December 2019. The primary outcomes were the one-time success rate of ligation, major bleeding rate, intraoperative dislodgement rate and operative time, which were compared between the two groups. There were 224 patients involved in this study, of whom 103 were in the Abs-o-lock® group and 121 were in the Hem-o-lok® group. The one-time success rate of ligation was 96.5% in the Abs-o-lock® group and 98.9% in the Hem-o-lok® group (p < 0.05). No major bleeding occurred in either group. The intraoperative dislodgement rate did not significantly differ between the two groups (p = 1.0). The operative time consumed in the Abs-o-lock® group was much longer than that in the Hem-o-lok® group for subgroups of resection of extralobar sequestration (p < 0.05), lobectomy (p < 0.05) and segmentectomy (p < 0.05). Compared to Hem-o-lok® clips, it is feasible and safe to apply 5-mm absorbable clips for vessel sealing during thoracoscopic anatomical lung resection in younger children.

Congenital Lung Malformations: Pulmonary Function Assessment Before and After Surgery

BackgroundThe management of asymptomatic congenital lung malformations (CLM) is debated, and pulmonary function of patients with CLM is seldom discussed. Short-term respiratory outcome in patients operated for CLM was assessed in order to better define surgical impact. MethodsA retrospective study on patients operated for CLM between 2012 and 2021 was performed. Respiratory function was assessed with tidal breath analysis (TBA) in spontaneous sleep within 2 years of life. Patients with comorbidities affecting pulmonary function were excluded. Three variables were studied: tPTEF/tE (time to peak tidal expiratory flow/total expiratory time), tV (tidal volume), RR (respiratory rate). Results were assessed as z-score (normal ±1.64) and expressed as median (range). Pre- and post-operative results were compared, as well as post-operative results of disease and surgery type subgroups. ResultsThirty-nine patients were included. Median pre- and post-operative tPTEF/tE were normal (pre: −0.32 (−2.12; +1.56); post: −0.18 (−1.62; +2.43)). Patients with extralobar sequestration had pre-operative high median RR, which improved after sequestrectomy (pre: +1.99 (−2.49; +7.43); post −0.22 (−3.01; +1.18)). All groups had reduced pre-operative median tV, which restored after surgery (pre: −2.15 (−9.75; +0.91); post: −0.35 (−6.65; +3.14)). Patients undergoing lobectomy and patients with intralobar sequestration showed greater improvement. Median post-operative TBA results were normal for all sub-groups except for reduced tV in patients operated with thoracotomy (−2.27 (−6.12; +5.26)). ConclusionsPatients with clinically asymptomatic CLM can have pathologic pulmonary function tests, which improve after surgery. These results add to the elements in favor of an interventional approach. Level of evidenceIII, Treatment study.

Intralobar pulmonary sequestration: robotical resection using indocyanine green

Pulmonary sequestration (PS) is a relatively rare congenital lung anomaly. There are two different subtypes of PS, intralobar and extralobar sequestration. The majority of cases is constituted by intralobar sequestration. Here, we report a case of a 39-year-old female with intralobar sequestration, which was successfully resected by robotic-assisted surgery.

Morphologic Features in Congenital Pulmonary Airway Malformations and Pulmonary Sequestrations Correlate With Mutation Status: A Mechanistic Approach to Classification.

Congenital pulmonary airway malformations (CPAMs) have a range of morphologies with varying cyst sizes and histologic features (types 1 to 3). Evidence suggested they arise secondary to bronchial atresia, however, we recently showed that cases with type 1 and 3 morphology are driven by mosaic KRAS mutations. We hypothesized that 2 distinct mechanisms account for most CPAMs: one subset is secondary to KRAS mosaicism and another is due to bronchial atresia. Cases with type 2 histology, similar to sequestrations, would be related to obstruction and therefore negative for KRAS mutations regardless of cyst size. We sequenced KRAS exon 2 in type 2 CPAMs, cystic intralobar and extralobar sequestrations, and intrapulmonary bronchogenic cysts. All were negative. Most sequestrations had a large airway in the subpleural parenchyma adjacent to the systemic vessel, anatomically confirming bronchial obstruction. We compared morphology to type 1 and 3 CPAMs. On average, type 1 CPAMs had significantly larger cysts, but there remained substantial size overlap between KRAS mutant and wild-type lesions. Features of mucostasis were frequent in sequestrations and type 2 CPAMs, while their cysts were generally simple and round with flat epithelium. Features of cyst architectural and epithelial complexity were more common in type 1 and 3 CPAMs, which rarely showed mucostasis. Similarity in histologic features among cases that are negative for KRAS mutation support the hypothesis that, like sequestrations, the malformation of type 2 CPAMs is related to obstruction during development. A mechanistic approach to classification may improve existing subjective morphologic methods.

Impact of Previous Infection on Perioperative Outcomes of Thoracoscopic Lobectomy for Congenital Lung Malformation.

Aim: To evaluate the impact of previous infection on perioperative outcomes in patients undergoing thoracoscopic lobectomy for congenital lung anomalies. Methods: This was a single-institution retrospective observational study for which patients who underwent thoracoscopic lobectomy for congenital lung disease between 2009 and 2021 were enrolled, and patients with extralobar sequestration were excluded. Patient background and data related to the surgery were compared between patients who had an infection before surgery (Group 1) and those who did not (Group 2). Results: This study included 34 patients, 13 in Group 1 and 21 in Group 2. The sex-based distribution and pathological diagnosis were similar between the two groups. Malformations were prenatally diagnosed in 1 patient in Group 1 (7.7%) and 18 patients in Group 2 (86%; P < .001). The median age and weight at the time of the procedure and procedure duration were comparable between the two groups. The amount of blood loss was significantly higher in Group 1 (60 mL) than in Group 2 (20 mL; P = .0042). Four patients in Group 2 required reoperation due to air leakage, pyothorax, and cardiac tamponade, whereas none of the Group 1 patients required reoperation (P = .12). No conversion to thoracotomy was required in either group. The duration of postoperative admission was similar between the two groups (Group 1: 6 days versus Group 2: 6 days; P = .14). Conclusions: Preceding infection increased the amount of bleeding during thoracoscopic lobectomy but had little effect on other outcomes.

The long-term outcome following thoraco-amniotic shunting for congenital lung malformations

Aim of the StudyInsertion of a thoraco amniotic shunt (TAS) during fetal life is a therapeutic option where there is a high risk of death secondary to large congenital lung malformations (CLM). The aim of this study is to present our center's long-term experience. MethodsRetrospective single center review of the period (Jan 2000–Dec 2020). We included all fetuses that underwent TAS insertion for CLM with detailed analysis of those live newborns managed in our center. Data are quoted as median (range). Main resultsThirty one fetuses underwent 37 TAS insertions at a 25 (20–30) weeks gestational age. This was successful on 1st attempt in 30 (97%) fetuses. In 6 cases a 2nd shunt was required at 6.5 (2–10) weeks following the 1st insertion. Twenty-eight survived to be born.Sixteen (9 male) infants were delivered in our center at 39 (36–41) weeks gestational age and birth weight of 3.1 (2.6–4.2) kg. All infants underwent surgery at 2 (0–535) days (emergency surgery, n = 9; expedited n = 4; elective surgery, n = 3). Final histopathology findings were CPAM Type 1 (n = 14, n.b. associated with mucinous adenocarcinoma, n = 1), CPAM Type 2 (n = 1) and an extralobar sequestration (n = 1). Postoperative stay was 16 (1–70) days with survival in 15/16 (94%). One infant died at 1 day of life secondary to a combination of pulmonary hypoplasia and hypertension. Median follow up period was 10.7 (0.4–20.4) years. Nine (60%) children developed a degree of chest wall deformity though none have required surgical intervention. Clinically, 14/15 (93%) have otherwise normal lung function without limitations of activity, sporting or otherwise. One child has a modest exercise limitation (FVC – 70% predicted). ConclusionsTAS insertion is associated with high perinatal survival and should be considered in fetuses at risk of hydrops secondary to large cystic lung malformation. Their long term outcome is excellent although most have a mild degree of chest wall deformity.

VATS surgical anatomical resection of bronchopulmonary sequestration presenting as chest sepsis

BackgroundBronchopulmonary sequestration (BPS) is a malformation of the lungs resulting in lung tissue lacking direct communication to the tracheobronchial tree. Most cases demonstrate systemic arterial blood supply from the descending thoracic aorta, the abdominal aorta, celiac axis or splenic artery and venous drainage via the pulmonary veins with occasional drainage into azygos vein. BPS is considered a childhood disease and accounts for 0.15–6.40% of congenital pulmonary malformations. BPS is divided into intralobar sequestrations (ILS) and extralobar sequestrations (ELS) with ILS accounting for 75% of all cases.MethodsHere we present our 11-year experience of dealing with BPS; all cases presented with recurrent chest sepsis in young-late adulthood regardless of the type of pathological sequestration. The surgical technique employed was a minimally invasive video-assisted thoracoscopic anterior approach (VATS).ResultsBetween May 2010 and September 2021, we have operated on nine adult patients with bronchopulmonary sequestration who presented late with symptoms of recurrent chest sepsis. Most patients in the cohort had lower lobe pathology, with a roughly even split between right and left sided pathology. Moreover, the majority were life-long never smokers and an equal preponderance in males and females. The majority were extralobar sequestrations (56%) with pathological features in keeping with extensive bronchopneumonia and bronchiectasis. There were no major intra-operative or indeed post-operative complications. Median length of stay was 3 days.ConclusionsDissection and division of the systemic feeding vessel was readily achievable through a successful anterior VATS approach, regardless of the type of sequestration and without the use of pre-operative coiling of embolization techniques. This approach gave excellent access to the hilar structures yet in this pathology, judicious and perhaps a lower threshold for open approach should be considered.

Extralobar Sequestration With a Feeding Vessel Arising from the Pulmonary Artery

Extralobar Sequestration With a Feeding Vessel Arising from the Pulmonary Artery

Uniportal Video-Assisted Thoracoscopic Resection and Lobectomy for Infants With Pulmonary Sequestration: Case Series and Initial Experience

Background: The present study aimed to evaluate the safety and feasibility of uniportal video-assisted thoracoscopic surgery (U-VATS) for infants with pulmonary sequestration (PS).Methods: From January 2019 to July 2020, 19 infants with PS were admitted to a provincial hospital in the Fujian Province of China. A 1.5-cm utility port was created in the fifth intercostal space at the anterior axillary line. A rigid 30° 5-mm optic thoracoscope was used for vision, and two or three instruments were utilized through the port. Surgical options include standard lobectomy, wedge resection, and resection of the extralobar sequestration. Only one intercostal space was entered, and a chest tube was inserted through the same skin incision if necessary.Results: The procedure was successful in all patients with an average operation duration of 58.3 ± 31.5 min. The length of post-operative hospital stay was 5.4 ± 1.5 days, and no post-operative deaths or serious complications were observed. The mean post-operative drainage volume was 164.6 ± 45.9 mL, and the mean post-operative thoracic tube indwelling duration was 5.5 ± 1.0 days. No intraoperative conversion, surgical mortality, or major complications were identified among the patients.Conclusion: Our preliminary experience presented a series of U-VATS lobectomy, wedge resection, and resection of the PS for infants with satisfactory perioperative results.

Extrathoracic lung sequestration in newborns: cases report

BACKGROUND: Extrathoracic lung sequestration is a rare variant of a developmental malformation that can be diagnosed prenatally and should be included in the differential diagnosis of abdominal tumor-like formations.&#x0D; CASES REPORT: From 1996 to 2020, 70 children with confirmed lung sequestration were treated at the Children's City Hospital No. 1 in St. Petersburg. Of these, 29 had intralobar sequestration, and 41 had extralobar sequestration. Four of these patients were diagnosed with a rare extrathoracic (intraabdominal) form. All four were born full-term without clinical manifestations of the defect. Ultrasound examination of all children revealed a solid formation up to 3 cm in diameter, located in the upper left quadrant of the abdominal cavity or retroperitoneal space in the projection of the left adrenal gland. Due to the location of the formation and the inability to visualize the aberrant arterial vessel during ultrasound examination, the first two patients were differentially diagnosed with an adrenal tumor (neuroblastoma). The cancer markers were negative, and adrenal cortex hormones were normal. The children underwent multispiral computed tomography-angiography to clarify the diagnosis. In both cases, the diagnosis of intraabdominal extrapulmonary sequestration with a feeding arterial vessel extending from the thoracic aorta was confirmed. Two children, previously treated in the last two years, were diagnosed by the ultrasound examination results that enabled the visualization of an aberrant arterial vessel feeding the sequester. Indications for surgical treatment have been set. The parents of one child refused treatment. Three children underwent sequestration removal (two by laparoscopic access, one had a laparotomy). Histologically, extrathoracic sequestration in combination with cystoadenomatosis was confirmed. The postoperative period proceeded without complications.&#x0D; RESULTS: Long-term treatment results were observed in all operated patients for a period of four to 10 years. The assessment was performed based on complaints and ultrasound data. All patients matured and developed according to their ages.&#x0D; CONCLUSIONS: Extrathoracic lung sequestration is a rare type of defect that is subject to surgical treatment. This pathology should be included on the spectrum of differential diagnosis of tumorous formations of the abdominal cavity and retroperitoneal space in newborns. The main methods for postnatal diagnosis of the defect are ultrasound and multispiral computed tomography-angiography.

A LUNG WITHIN A LUNG? A CASE REPORT OF MASSIVE HEMOPTYSIS SECONDARY TO PULMONARY SEQUESTRATION

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Bronchopulmonary sequestration (BPS), is defined as a non-functional mass of lung tissue with systemic arterial blood supply, but without normal tracheobronchial communication. BPS often presents in adulthood with recurrent pulmonary infections and even fatal hemoptysis. We present a case of a 53-year-old African American male with large volume hemoptysis while hiking in upstate New York, who was found to have right lower lobe intralobar pulmonary sequestration. CASE PRESENTATION: A 53-year-old African American male with a past medical history of hypertension, hyperlipidemia, and diabetes mellitus presented with a subacute cough for one month and one episode of large volume hemoptysis while hiking. Review of systems was negative for fever, chills, chest pain, pleurisy, history of tuberculosis, weight loss, and anticoagulation use. He admitted to recreational marajuana use, but denied smoking cigarettes. Initially he was tachypneic, saturating 91% on room air, but hemodynamically stable. Computed tomography angiography (CTA) revealed right lower lobe intralobar pulmonary sequestration with internal locules of air suggesting superimposed infection. Flexible bronchoscopy revealed fresh blood in the right mainstem and lower lobe. Thoracotomy was followed by a lower lobectomy, which confirmed the diagnosis as it demonstrated an aberrant arterial supply from the descending thoracic aorta. There was no envelopment of pleura around the lung fed by the large pulsatile artery. DISCUSSION: Pulmonary airway malformations are the most common type of congenital abnormalities of the lower respiratory tract, diagnosed 1 in 10,000 to 35,000 live births [1]. An intralobar sequestration (IS) is located in a normal lobe and an extralobar sequestration (ES) is outside, with both having their own visceral pleura. Hybrid lesions are most common and have features of both. ES presents early with associated congenital malformations, whereas IS presents later with hemoptysis and recurrent pulmonary infections [2]. BPS can appear as other pathologies on CT such as a mass lesion, cyst, cavity lesion and localized emphysema [3,4]. Lobectomy should be prompt to prevent complications, such as fatal hemoptysis [5]. Shorter recovery times have been noted with video-assisted thoracoscopic surgery (VATS) [6,7]. Postoperative complications include empyema, hemoptysis, prolonged air leak, and fistula formation. Berna et al described their surgical approach in 25 patients in 2011, and all patients were doing well at long-term follow-up [7]. CONCLUSIONS: BPS can be fatal despite its rarity and innocent presentation. Radiographic ambiguity and nonspecific symptoms may delay appropriate management. It is imperative to include BPS on the initial query when managing hemoptysis. In the era of COVID-19, where hemoptysis may herald a deadly disease, understanding the broad differential of hemoptysis expedites appropriate management. REFERENCE #1: [1] Durell J, Thakkar H, Gould S, Fowler D, Lakhoo K. Pathology of asymptomatic, prenatally diagnosed cystic lung malformations. J Pediatr Surg. 2016;51(2):231-235. doi:10.1016/j.jpedsurg.2015.10.061 REFERENCE #2: [2] Van Raemdonck D, De Boeck K, Devlieger H, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg. 2001;19(4):388-395. doi:10.1016/s1010-7940(01)00603-0 REFERENCE #3: [3] Wei Y, Li F. Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40(1):e39-e42. doi:10.1016/j.ejcts.2011.01.080[4] Qi W, Zhao J, Shi G, Yang F. Intralobar pulmonary sequestration displayed as localized emphysema on computed tomography image. J Cardiothorac Surg. 2017;12(1):83. Published 2017 Sep 8. doi:10.1186/s13019-017-0646-9[5] Rubin EM, Garcia H, Horowitz MD, Guerra JJ Jr. Fatal massive hemoptysis secondary to intralobar sequestration. Chest. 1994 Sep;106(3):954-5. doi: 10.1378/chest.106.3.954. PMID: 8082388.[6] Sun X, Xiao Y. Pulmonary sequestration in adult patients: a retrospective study. Eur J Cardiothorac Surg. 2015;48(2):279-282. doi:10.1093/ejcts/ezu397[7] Polaczek, M., Baranska, I., Szolkowska, M., Zych, J., Rudzinski, P., Szopinski, J., Orlowski, T., & Roszkowski-Sliz, K. (2017). Clinical presentation and characteristics of 25 adult cases of pulmonary sequestration. Journal of thoracic disease, 9(3), 762–767. https://doi.org/10.21037/jtd.2017.03.107 DISCLOSURES: No relevant relationships by Christian Castaneda, source=Web Response no disclosure on file for Rammohan Gumpeni; No relevant relationships by Sophia Ji, source=Web Response No relevant relationships by Parmjyot Singh, source=Web Response No relevant relationships by Anthony Smith, source=Web Response

Hybrid minimally invasive treatment of intralobar pulmonary sequestration: a single-centre experience.

Pulmonary sequestrations are rare congenital malformations. They are often located in the lower lobes, and they are supplied by an aberrant systemic vessel arising from the thoracic aorta or abdominal arteries. These pulmonary malformations are divided into intra- and extralobar sequestrations, depending on the respective lack or presence of an independent pleural covering. Pulmonary sequestration can be asymptomatic or lead to recurrent pulmonary infections. The goal of this study was to analyse the feasibility and safety of a hybrid sequential approach. We report a small series of intralobar pulmonary sequestrations, from November 2017 to December 2018, successfully treated with a hybrid minimally invasive approach consisting of endovascular embolization of the aberrant arterial branch followed by video-assisted thoracoscopic lobectomy the day after. Thoracic pain following endovascular embolization was noted in all cases. Patients were discharged early in the absence of major postoperative complications. Prolonged air leak was observed in only 1 case. Despite the presence of sequestration-related pulmonary inflammation, in our experience, hybrid treatment for intralobar pulmonary sequestration is a safe and reproducible approach in terms of postoperative complications and hospital stay.

Extralobar pulmonary sequestrations hiding congenital diaphragmatic defects: A case series

Background: Association between pulmonary sequestration and diaphragmatic hernia is well known. Extralobar sequestrations are masses of the non-functioning lung, surrounded by separate pleura, without bronchial communication, and with a systemic arterial blood supply. They may be placed in the thorax, within the diaphragm, or rarely in a sub-diaphragmatic position. Case Series: We present three cases of extra-lobar extra-thoracic pulmonary sequestrations associated with different types of diaphragmatic defects. In none of the three cases, the diaphragmatic defect was detected prenatally. Conclusion: Pulmonary sequestration may be involved in the embryological origin of the diaphragmatic defect. Simultaneously, it acts as an anatomical barrier and prevents the herniation of the abdominal content into the thorax. If extralobar pulmonary sequestration is diagnosed prenatally, a coexistent diaphragmatic hernia should always be considered.

Revising the classification of lung sequestrations

PurposesThe classification of lung sequestrations distinguishes between extralobar and intralobar types, according to their venous drainage – systemic vs pulmonary – and the presence or absence of independent pleura. However, imaging, surgical and/or pathological findings often differ from this description.The objectives of this article are to quantify the percentage of lung sequestrations that do not fit the classic description of extra- and intralobar types and to evaluate the accuracy of the currently used classification. MethodsA retrospective search identified all children with a confirmed lung sequestration diagnosed and treated in our Hospital over the last 10 years. Two senior pediatric radiologists reviewed their contrast-enhanced computed tomography chest scans and evaluated the main anatomical features that define sequestrations, including pleura, arterial and venous pattern, airways and lung parenchyma. We compared the imaging-, surgical- and pathological findings to those described for extra- and intralobar sequestrations. Results25 children (20 M, 5 F) conform the series. Only 13 lesions (52%) filled all criteria described for an extra- or intralobar sequestration. The remaining 12 lesions (48%) had at least one differing criteria, including incomplete independent pleura (n = 2; 8%), mixed systemic and pulmonary arterial supply (n = 1; 4%) or venous drainage (n = 3; 12%), normal connection to airway (n = 1; 4%) and/or coexistent congenital lung anomalies (n = 11; 44%). ConclusionLung sequestrations seem to represent a spectrum of anomalies rather than separated entities. Therefore, a detailed description of their main anatomical features could be more relevant for clinicians and surgeons that the rigid distinction in intra- and extralobar sequestration currently applied.

Concurrent bronchopulmonary foregut malformations: a rare case of right-sided extralobar pulmonary sequestration and bronchogenic cyst

BackgroundBronchopulmonary foregut malformations are rare congenital malformations. It is extremely rare to have malformations that occur simultaneously. There is literature to show that extralobar sequestration is associated with other congenital anomalies, most commonly diaphragmatic hernias, and also with other bronchopulmonary foregut malformations (e.g., extralobar sequestration and congenital pulmonary airway malformations). However, very few case reports were found that reported extralobar sequestration and foregut duplication cysts and only one report of a right-sided complex foregut malformation with pulmonary sequestration.Case presentationWe present a case of a 3-month-old male infant with a prenatal diagnosis of a cystic lung lesion who, after developing symptoms of respiratory distress, was found to have concurrent right-sided extralobar pulmonary sequestration and a mediastinal bronchogenic cyst.ConclusionsThe concurrent occurrence of these malformations in one patient could help support the theory that these malformations result from an early error in development during the time when both the lung buds and foregut are developing simultaneously.

Congenital pulmonary airway malformation and bronchopulmonary sequestration in infants - a rare diagnosis

Herein is described the case of a two-month-old female infant with recurrent respiratory infections, admitted to the Pediatric Emergency Department with productive cough, respiratory distress, and fever. Chest radiography showed an heterogenous hypotransparent image in the left pulmonary field and contralateral deviation of the mediastinum. Angiotomography revealed bilateral bronchopulmonary sequestration and a systemic vessel contributing to area irrigation. The patient was submitted to vessel occlusion and lacquering, left inferior lobectomy, and resection of a right lower lobe segment. Histological examination confirmed intralobar bronchopulmonary sequestration and type 2 congenital pulmonary airway malformation in the left lung, and extralobar bronchopulmonary sequestration in the right lung.With this report, the authors intend to emphasize the importance of clinical suspicion of congenital anomalies in infants with recurrent respiratory infections.